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Haematological Malignacies Flashcards

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1
Q

Define myelomas

A

Proliferation of plasma cells

  • terminal cell of B lymphocyte
  • release immunoglobulins - non-functioning
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2
Q

Features of myelomas

A

Bone marrow infiltration -> anaemia/thrombocytopenia
Cause bone and renal disease
Can form solid tumours of plasma cells (plasmacytomas)
Impaired immune system
Treatable but not curable

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3
Q

Myeloma presentation

A 
Bones
- generalised osteopenia
- pathological fractures
Hypercalcaemia
- polyuria, polydipsia, abdo pain, constipation, lethargy, confusion
Bone marrow infiltration
- anaemia
- thrombocytopenia
- neutropenia
Infections
Hyperviscotisy syndrome
- visual disturbance 
- headache
- - - -
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4
Q

What define myeloma

A 
Myeloma defining events
S - 60% BM plasmacytosis
Li - light chain ratio > 100
M - > 1 focal lesions on MRI > 5 mm
Evidence of end organ damage
C - calcium elevation > 2.75
R - renal impairment Cr>117
A - anaemia Hb < 100
B - bone lesion
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5
Q

Define haemopoiesis

A

Formation of blood cells from a hemopoietic stem cell

  • cell isolated from blood or bone marrow can renew itself
  • able to differentiate to a variety of specialised cells
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6
Q

Where does haemopoiesis go wrong

A 
Loss of normal tight controls on haemopoiesis
- too many cells proliferating 
- don't apoptose/die when should
- don't differentiate (maturation arrest)
Myeloid 
- acute myeloid leukaemia
- chronic myeloproliferative neoplasms
Lymphoid
- acute lymphoblastic leukaemia
- chronic lymphoblastic leukaemia
- lymphoma
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7
Q

Leukaemia vs lymphoma

A

Leukaemia - affecting mainly bone marrow with or without released circulating neoplastic cells in blood
Lymphoma - predominantly nodal or organ-based

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8
Q

Define leukaemia

A

Cancer of stem cell line in bone marrow

Increase in number of white blood cells

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9
Q

Classification of leukaemia

A

Myeloid vs lymphoid
- depends on which lineage is involved
Acute vs chronic
- based on maturity of cells
- acute has proliferation of immature blast cells
- chronic has proliferation of more mature cells
- pace of disease faster in chronic

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10
Q

Define myeloproliferative neoplasms

A

Chronic proliferation of myeloid cells
Red = polycythaemia
Platelets = thrombocythemia
White = leukaemia (CML)

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11
Q

Features of bone marrow

A

Where blood cells develop

Mostly found in pelvis, vertebrae, ribs and sternum

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12
Q

Development of blood cells

A

Pluripotent haematopoietic stem cells
- undifferentiated cells that have the potential to become - myeloid stem cells, lymphoid stem cells and dendritic cells
Red blood cells
- develop from reticulocytes that come from myeloid stem cells
- survive up to 3 months
Platelets
- made by megakaryocytes
- lifespan of 10 days
White blood cells
- myeloid stem cells become promyelocytes
- monocytes then macrophages
- neutrophils
- eosinophils
- mast cells
- basophils
- lymphocytes come from lymphoid stem cells - become B and T cells
- B lymphocytes mature in the bone marrow and differentiate into plasma or memory B cells
- t lymphocytes mature in thymus gland and differentiate into CD4 cells (T helper), CD8 cells (cytotoxic T cells) and natural killer cells

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13
Q

Features of blood films

A

Anisocytosis - variation in size of red blood cells
- myelodysplastic syndrome as well as anaemia
Target cells - central pigmented area surrounded by pale area surrounded by ring of thicker cytoplasm
- iron deficiency anaemia and post-splenectomy
Heinz bodies - individual blobs seen inside RBC caused by denatured globin
- G6PD and alpha-thalassaemia
Howell-Jolly bodies - individual blobs of DNA material seen in RBC, normally removed by spleen
- post-splenectomy and severe anaemia
Reticulocytes - larger immature RBC, still have RNA material
- haemolytic anaemia
Schistocytes - fragments of RBCs
- haemolytic uraemic syndrome, DIC, thrombotic thrombocytopenia purpura, metallic heart valves, haemolytic anaemia
Sideroblasts - immature RBC that contain blobs of iron, occur when bone marrow unable to incorporate iron into haemoglobin
- myelodysplasic syndrome
Smudge cells - ruptured WBC, occur during process of preparing the blood film due to aged or fragile WBC
- chronic lymphocytic leukaemia
Spherocytes - spherical RBCs
- autoimmune haemolytic anaemia or hereditary spherocytosis

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14
Q

Define anaemia

A

Low level of haemoglobin

  • women 120-165
  • men 130-180
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15
Q

Causes of microcytic anaemia

A

TAILS

  • thalassaemia
  • anaemia of chronic disease
  • iron deficiency anaemia
  • lead poisoning
  • sideroblastic anaemia
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16
Q

Causes of normocytic anaemia

A

3As and 2Hs

  • acute blood loss
  • anaemia of chronic disease
  • aplastic anaemia
  • haemolytic anaemia
  • hypothyroidism
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17
Q

Causes of macrocytic anaemia

A 
Megaloblastic
- result of impaired DNA synthesis preventing cell from dividing normally
- B12 or folate deficiency
Normoblastic
- alcohol
- reticulocytosis
- hypothyroidism
- liver disease
- azathioprine
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18
Q

Symptoms of anaemia

A 
Fatigue
SOB
Headaches
Dizziness
Palpitations
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19
Q

Specific symptoms of iron deficiency anaemia

A

Pica - dietary cravings

Hair loss

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20
Q

Signs of anaemia

A 
General
- pale skin
- conjunctival pallor
- tachycardia
- raised RR
Specific
- koilonychia, angular cheilitis, atrophic glossitis - iron deficiency
- jaundice - haemolytic
- bone deformities - thalassaemia
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21
Q

Ix for anaemia

A 
Haemoglobin
Mean Cell Volume (MCV)
B12
Folate
Ferritin
Blood film
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22
Q

Causes of iron deficiency anaemia

A 
Blood loss - most common in adults
- menorrhagia
- GI tract cancer
Dietary insufficiency - most common in growing children
Poor iron absorption
Increased requirements during pregnancy
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23
Q

Features of iron deficiency

A

Ferritin - form of iron when deposited and stored in cells
- low suggestive of iron deficiency
- high is difficult to interpret as related to inflammation
Serum iron - varies throughout day
Total iron binding capacity - marker for how much transferrin is in blood
Transferrin saturation - good indication of total iron in body

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24
Q

Mx of iron deficiency anaemia

A

Blood transfusion
Iron infusion
Oral iron

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25
Pathophysiology of pernicious anaemia
Autoimmune condition | Antibodies form against parietal cells or intrinsic factor preventing absorption of vitamin B12
26
Symptoms of B12 deficiency
Peripheral neuropathy with numbness or paraesthesia Loss of vibration sense or proprioception Visual changes Mood or cognitive changes
27
Ix for B12 deficiency
Testing for auto-antibodies - intrinsic factor antibody - 1st line - gastric parietal cell antibody
28
Mx of B12 deficiency anaemia
Dietary deficiency - oral replacement - cyanocobalamin | Pernicious anaemia - 1mg IM hydroxycobalamin 3x weekly for 2 weeks, then every 3 months
29
Define autoimmune haemolytic anaemia
Antibodies created against patients RBCs | Leads to destruction of RBC
30
Types of autoimmune haemolytic anaemia
``` Warm - more common - haemolysis occurs at normal or above normal temperatures - idiopathic Cold - antibodies attach at lower temperatures causing RBC to clump together - agglutination -> destruction of RBC - secondary to other conditions - lymphoma - leukaemia - SLE - EBV - CMV - HIV ```
31
Mx of autoimmune haemolytic anaemia
Blood transfusions Prednisolone Rituximab - monoclonal antibody against B cells Splenectomy
32
Define thalassaemia
Genetic defect in protein chains that make up haemoglobin - defects in alpha-globin -> alpha thalassaemia - chromosome 16 - defects in beta-globin -> beta thalassaemia - chromosome 11 Both autosomal recessive
33
Clinical features of thalassaemia
``` Microcytic anaemia (low mean corpuscular volume) Fatigue Pallor Jaundice Gallstones Splenomegaly Poor growth and development Pronounced forehead and malar eminences ```
34
Ix for thalassaemia
FBC - microcytic anaemia Haemoglobin electrophoresis DNA testing
35
Complications of thalassaemia
Iron overload - faulty RBC, recurrent transfusions and increased iron absorption Monitor serum ferritin Mx involves limiting transfusions and iron chelation
36
Mx of alpha-thalassaemia
``` Monitoring the full blood count Monitoring for complications Blood transfusions Splenectomy may be performed Bone marrow transplant can be curative ```
37
Types of beta-thalassaemia
Minor - one normal and one abnormal gene - causes mild microcytic anaemia Intermedia - two defective genes or one defective and one deletion - more significant microcytic anaemia Major - homozygous for deletion genes - severe anaemia and failure to thrive in early childhood - causes severe microcytic anaemia, splenomegaly and bone deformities
38
Define sickle cell anaemia
Genetic condition that causes sickle shaped red cells
39
Pathophysiology of sickle cell anaemia
Foetal haemoglobin replaced by haemoglobin A at around 6 weeks Patients with sickle cell have abnormal haemoglobin variant S - causes red blood cells to sickle Autosomal recessive Abnormal gene for beta-globin on chromosome 11
40
Complications of sickle cell anaemia
``` Anaemia Increased risk of infection Stroke Avascular necrosis in large joints such as the hip Pulmonary hypertension Painful and persistent penile erection (priapism) Chronic kidney disease Sickle cell crises Acute chest syndrome ```
41
Mx of sickle cell
Avoid dehydration and other triggers of crises Ensure vaccines are up to date Antibiotic prophylaxis to protect against infection with penicillin V (phenoxymethypenicillin) Hydroxycarbamide can be used to stimulate production of fetal haemoglobin (HbF). Fetal haemoglobin does not lead to sickling of red blood cells. This has a protective effect against sickle cell crises and acute chest syndrome. Blood transfusion for severe anaemia Bone marrow transplant can be curative
42
Define sickle cell crisis
Umbrella term for spectrum of acute crises related to the condition Occur spontaneously of triggered by stresses - infection - dehydration - cold - significant life events
43
Mx of sickle cell crisis
Have a low threshold for admission to hospital Treat any infection Keep warm Keep well hydrated (IV fluids may be required) Simple analgesia such as paracetamol and ibuprofen Penile aspiration in priapism
44
Types of leukaemia
Acute myeloid leukaemia Acute lymphoblastic leukaemia Chronic myeloid leukaemia Chronic lymphocytic leukaemia
45
Pathophysiology of leukaemia
Genetic mutation in one of the precursor cells leads to excessive production of single type of abnormal WBC Suppression of the cell lines -> pancytopenia - low RBC - anaemia - low WBC - leukopenia - low platelets - thrombocytopenia
46
Epidemiology of leukaemia
Under 5 and over 45 - ALL Over 55 - CLL Over 65 - CML Over 75 - AML
47
Presentation of leukaemia
``` Non-specific - need urgent FBC Fatigue Fever Failure to thrive (children) Pallor due to anaemia Petechiae and abnormal bruising due to thrombocytopenia Abnormal bleeding Lymphadenopathy Hepatosplenomegaly ```
48
Differential diagnosis of petechiae
Caused by thrombocytopenia - leukaemia - meningococcal septicaemia - vasculitis - idiopathic thrombocytopenia purpura - non-accidental injury
49
Ix for leukaemia
``` FBC - within 48 hrs Blood film LDH - raised but not specific CXR - infection or mediastinal lymphadenopathy Lymph node biopsy Lumbar puncture - if CNS involvement CT/MRI/PET for staging and assessing ```
50
Types of bone marrow biopsy
``` Bone marrow aspiration - taking liquid sample full of cells Bone marrow trephine - solid core sample of bone marrow - better assessment of cells and structure Bone marrow biopsy - usually taken from iliac crest - local anaesthetic and specialist needle - samples can be examined straight away ```
51
Define Acute Lymphoblastic Leukaemia
Malignant change in one the lymphocyte precursor cells - > acute proliferation of a single type of lymphocyte - usually B lymphocytes - > replace other cell types created by bone marrow - pancytopenia
52
Epidemiology of ALL
``` Most common cancer in children - peaks 2-4 years Also affects adults over 45 Associated with Downs syndrome Associated with Philadelphia chromosome translocation ```
53
Features of blood film of ALL
Blast cells - large cell with very large nucleus
54
Define chronic lymphocytic leukaemia
Chronic proliferation of single type of well differentiated lymphocyte - usually B lymphocytes
55
Epidemiology of CLL
Adults over 55 | Can transform into high-grade lymphoma - Richter's transformation
56
Clinical features of CLL
``` Asymptomatic Infection Anaemia Bleeding Weight loss Can cause warm autoimmune haemolytic anaemia ```
57
Blood film features of CLL
Smear or smudge cells | - aged or fragile WCC rupture leaving a smudge on the film
58
Features of chronic myeloid leukaemia
``` 3 phases - chronic - accelerated - blast Associated with Philadelphia chromosome ```
59
Feature of chronic phrase of CML
Lasts around 5 years | Often asymptomatic - patients diagnosed incidentally with raised WCC
60
Features of accelerated phase of CML
Occurs when abnormal blast cells take up high proportion of cells in bone marrow and blood Patients become symptomatic, develop anaemia and thrombocytopenia and become immunocompromised
61
Features of the blast phrase of CML
Involves high proportion of blast cells and blood Severe symptoms and pancytopenia Often fatal
62
Features of acute myeloid leukaemia
Many different types all with slightly different cytogenetic differences and differences in presentation
63
Epidemiology of AML
Most common acute leukaemia in adults Can present at any age - normally middle age onwards Can result from a transformation from myeloproliferative disorder - polycythaemia ruby vera or myelofibrosis
64
Features of blood film of AML
High proportion of blast cells | - Auer rods - rods inside cytoplasm
65
Mx of leukaemia
Mainly chemotherapy and steroids | Allogenic stem cell transplant
66
Complications of leukaemia
``` Failure Stunted growth and development in children Infections due to immunodeficiency Neurotoxicity Infertility Secondary malignancy Cardiotoxicity Tumour lysis syndrome ```
67
Pathophysiology of tumour lysis syndrome
Causes by release of uric acid from cells that are being destroyed by chemotherapy Uric acid can form crystals in interstitial tissue and tubules of kidneys
68
Mx of tumour lysis syndrome
Allopurinol or rasburicase used to reduce high uric acid levels
69
Define lymphomas
Group of cancers that affect lymphocytes inside lymphatic system Cancerous cells proliferate within lymph nodes -> lymphadenopathy
70
Categories of lymphoma
Hodgkin's - specific disease Non-Hodgkin's - encompasses all other lymphomas - B cell or T cell in origin - either low (aggressive, large cells seen) or high grade (small cells) - high grade tumours have high mortality in first few years but then good prognosis as less likely to reoccur
71
Epidemiology of Hodgkin's lymphoma
1 in 5 lymphomas Caused by proliferation of lymphocytes Bimodal age distribution with peaks around 20 and 75
72
Risk factors for Hodgkin's lymphoma
HIV EBV Autoimmune conditions - RA and sarcoidosis FHx
73
Clinical features of Hodgkin's lymphoma
``` Lymphadenopathy - enlarged lymph nodes - neck, axilla or inguinal region - non-tender and rubbery - pain in nodes when drinking Fatigue Itching Cough Shortness of breath Abdominal pain Recurrent infections B symptoms - fever - weight loss - night sweats ```
74
Ix of lymphoma
``` Lactate dehydrogenase - raised in Hodgkin's but not specific Lymph node biopsy - Reed-Sternberg cells - abnormally large B cells with multiple nuclei with nucleoli inside them - owl face CT, MRI or PET for stagin ```
75
Staging of lymphoma
Ann Arbor Staging used for all lymphomas Stage 1 - confined to one region of lymph nodes Stage 2 - in more than one region but on same side of diaphragm Stage 3 - affects lymph nodes both above and below the diaphragm Stage 4 - widespread involvement including non-lymphatic organs such as lungs or liver A - asymptomatic B - b symptoms present E - presence of extranodal site
76
Mx of Hodgkin lymphoma
Chemotherapy (ABVD - doxorubicin (Adriamycin), bleomycin, vinblastine and dacarbazine (DTIC)) and radiotherapy - aim to cure - usually successful - risk of relapse
77
Side effects of mx of Hodgkin lymphoma
``` Chemotherapy - leukaemia - infertility Radiotherapy - cancer - damage to tissues - hypothyroidism ```
78
Types of non-Hodgkin Lymphoma
Burkitt lymphoma - associated with EBV, malaria and HIV MALT lymphoma - affects mucosa-associated lymphoid tissue, usually around the stomach, a/w H.pylori Diffuse large B cell lymphoma - presents as rapidly growing painless mass in patients over 65 years
79
Risk factors for non-Hodgkin's lymphoma
``` HIV Epstein-Barr Virus H. pylori (MALT lymphoma) Hepatitis B or C infection Exposure to pesticides and a specific chemical called trichloroethylene used in several industrial processes Family history ```
80
Mx of follicular lymphoma
``` Stage 2A - radiotherapy - watch and wait if unable Stage 3/4 (advanced) - A - rituximab - S - rituximab with chemotherapy - cyclophosphamide, vincristine and prednisolone (CVP) - cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP) ``` Stem cell transplantation
81
Define myeloma
Cancer of the plasma cells | -> large quantities of single type of antibody being produced
82
Define multiple myeloma
Myeloma that affects multiple areas of the body
83
Define MGUS
Monoclonal Gammopathy of Undetermined Significance Excess of a single type of antibody or antibody components without other features of myeloma or cancer Often incidental finding in otherwise healthy person May progress to myeloma
84
Define smouldering myeloma
Progression of MGUS with higher levels of antibodies or antibody components Premalignant
85
Pathophysiology of myeloma
Genetic mutation causing a plasma cell to rapidly and uncontrollably multiply Produce one type of antibodies (immunoglobulins) One type (A, G, M, D and E) significantly abundant - monoclonal paraprotein - IgG more than 50% of the time Bence Jones protein can be found in the urine of many myeloma patients - subunit of the antibody - light chains
86
Complications of myeloma
Anaemia Myeloma bone disease Myeloma renal disease Hyperviscocity
87
Features of anaemia of myeloma
Cancerous plasma cells invade the bone marrow - bone marrow infiltration Suppression of development of other blood cell lines - anaemia - low RBC - neutropenia - low neutrophils - thrombocytopenia - low platelets
88
Features of myeloma bone disease
Result of increased osteoclast activity and suppressed osteoblast activity Metabolism of bone is imbalanced - more bone reabsorbed than constructed - cytokines released from plasma cells and stromal cells Commonly occurs in skull, spine, long bones and ribs - some areas of bone becomes thin - osteolytic lesions - lead to pathological fractures Osteoclast activity causes a lot of calcium to be released into blood - hypercalcaemia Development of plasmacytomas - individual tumours made up of cancerous plasma cells
89
Features of myeloma renal disease
Development of renal impairment - High levels of immunoglobulins (antibodies) can block the flow through the tubules - Hypercalcaemia impairs renal function - Dehydration - Medications used to treat the conditions such as bisphosphonates can be harmful to the kidneys
90
Features of hyperviscocity of myeloma
Viscosity increases due to increased proteins Leads to - easy bruising - easy bleeding - reduced or loss of sight due to vascular disease - purple discolouration to the extremities - heart failure
91
4 key features of myeloma
``` CRAB Calcium elevated Renal failure Anaemia - normocytic, normochromic Bone lesions/pain ```
92
Risk factors for myeloma
``` Older age Male Black African ethnicity Family history Obesity ```
93
Ix for myeloma
``` Suspect in anyone over 60 with persistent bone pain, particularly back pain or unexplained fractures FBC - low WCC Calcium - raised ESR - raised Plasma viscosity - raised ```
94
Testing for myeloma
``` B - Bence-Jones protein - request urine electrophoresis L - serum-free light chain assay I - serum immunoglobulins P - serum protein electrophoresis Bone marrow biopsy - confirm diagnosis Imaging - whole body MRI - whole body CT - skeletal survery ```
95
Features of myeloma on x-ray
Punched out lesions Lytic lesions Raindrop skull
96
Mx of myeloma
``` Aim of treatment to control disease - often takes relapsing-remitting course Chemotherapy - bortezomid - thalidomide - dexamethasone Stem cell transplantation VTE prophylaxis ```
97
Mx of myeloma bone disease
Bisphosphonates - suppress osteoclast activity Radiotherapy - improve pain Orthopaedic surgery - stabilise bones Cement augmentation - injecting cement into vertebral fractures/lesions to improve spine stability and pain
98
Complications of myeloma
``` Infection Pain Renal failure Anaemia Hypercalcaemia Peripheral neuropathy Spinal cord compression Hyperviscocity ```
99
Define myeloproliferative disorder
Type of bone marrow cancer Occur due to uncontrolled proliferation of a single type of stem cell Have the potential to progress and transform into acute myeloid leukaemia Associated with mutations of JAK2, MPL and CALR genes
100
Types of myeloproliferative disorders
Primary myelofibrosis - proliferation of hemopoietic stem cells Polycythaemia vera - proliferation of erythroid cell line Essential thrombocythemia - proliferation of megakaryocytic cell line
101
Define myelofibrosis
Proliferation of cell line leads to fibrosis of bone marrow Response to cytokines released by proliferating cells Haematopoiesis occurs in other areas such as liver and spleen (extramedullary haematopoiesis) -> hepatomegaly and splenomegaly -> portal hypertension
102
Presentation of myeloproliferative disorders
``` Initially asymptomatic Systemic symptoms - fatigue - weight loss - night sweats - fever Underlying complications - anaemia - except in polycythaemia - splenomegaly (abdo pain) - portal hypertension ( ascites, varices and abdo pain) - low platelets (bleeding and petechiae - thrombosis - raised RBC - low WCC (infections) ```
103
Key features of examination of polycythaemia vera
Conjunctival plethora - excessive redness Ruddy complexion Splenomegaly
104
FBC findings for myeloproliferative disorders
``` Polycythaemia vera - raised Hb Primary thrombocythemia - raised platelet count Myelofibrosis - anaemia - leucocytosis or leukopenia - thrombocytosis or thrombocytopenia - tear-dropped shaped RBCs, varying sized RBC (poikilocytosis) and blast cells on blood film ```
105
Ix for myeloproliferative disorders
Bone marrow biopsy - bone marrow aspiration dry due to fibrosis Testing for JAK2, MPL and CALR genes
106
Mx of primary myelofibrosis
Those with mild disease monitored Allogenic stem cell transplantation - curative but carries risk Chemotherapy - help control disease, improve symptoms and slow progression but not curative alone Supportive
107
Mx of polycythaemia ver
Venesection - used to keep haemoglobin in normal range Aspirin - reduce thrombosis risk Chemotherapy - control disease
108
Mx of essential thrombocythaemia
Aspirin - reduce risk of thrombus formation | Chemotherapy - control disease
109
Features of myelodysplastic syndrome
Caused by myeloid bone marrow cells not maturing properly and producing healthy blood cells Causes low level of blood components that originate from the myeloid cell line - anaemia - neutropenia - thrombocytopenia Increased risk of transforming into acute myeloid leukaemia
110
Epidemiology of myelodysplastic syndromes
More common in patients over 60 | Patients who have previously had treatment with chemotherapy and radiotherapy
111
Clinical features of myelodysplastic syndromes
May by asymptomatic - incidentally diagnosed on FBC Present with symptoms of - anaemia - fatigue, pallor or SOB - neutropenia - frequent of severe infections - thrombocytopenia - purpura or bleeding
112
Ix of myelodysplastic syndromes
FBC abnormal Blasts on blood film Diagnosis confirmed by bone marrow aspiration and biopsy
113
Mx of myelodysplastic syndromes
Watchful waiting Supportive treatment with blood transfusions if severely anaemic Chemotherapy Stem cell transplantation
114
Define thrombocytopenia
Low platelet count
115
Causes of thrombocytopenia
``` Problems with production - sepsis - B12 or folic acid deficiency - liver failure - leukaemia - myelodysplastic syndrome Problems with destruction - medications - sodium valproate, methotrexate, antihistamines, PPIs - alcohol - immune thrombocytopenic purpura - heparin-induced - haemolytic uraemic syndrome ```
116
Presentation of thrombocytopenia
Mild may be asymptomatic Spontaneous bruising and prolonged bleeding times - nosebleeds, bleeding gums, heavy periods, blood in urine/stools
117
Ddx of abnormal or prolonged bleeding
Thrombocytopenia Haemophilia Von Willebrand disease DIC
118
Define immune thrombocytopenic purpura
Antibodies created against platelets - destruction of platelets - low platelet count
119
Mx of ITP
Prednisolone IV immunoglobulins Rituximab Splenectomy
120
Define thrombotic thrombocytopenic purpura
Tiny blood clots develop throughout small vessels of body | - using up platelets
121
Pathophysiology of TTP
Problem with ADAMTS13 protein - normally inactivated von Willebrand factor reducing platelet adhesion Shortage of protein leads to von Willebrand factor overactivity Clots break up RBC -> haemolytic anaemia
122
Mx of TTP
Referral to haematologist Plasma exchange Steroids Rituximab
123
Pathophysiology of heparin induced thrombocytopenia
Development of antibodies against platelets in response to heparin exposure - target platelet factor 4 - anti-PF4/heparin antibodies Bind to platelets and activate clotting mechanisms -> thrombosis Breakdown of platelets -> thrombocytopenia
124
Mx of heparin induced thrombocytopenia
Diagnosis by HIT antibody testing Stop heparin Use alternative anticoagulant
125
Features of von Willebrand disease
Most common inherited cause of abnormal bleeding Manly autosomal dominant Deficiency, absence or malfunctioning of glycoprotein von Willebrand factor
126
Mx of von Willebrand factor
``` Desmopressin - stimulates release of VWF VWF infusion Factor VIII Mx of heavy periods - tranexamic acid - mefanamic acid - norethisterone - COCP - Mirena coil - hysterectomy ```
127
Features of haemophilia A and B
A caused by deficiency of factor VIII B caused by deficiency of factor IX X-linked recessive - much more common in males
128
Presentation of haemophillia
``` Spontaneous haemorrhage - hemarthrosis - bleeding into muscles Presentation in neonatal/early period - intracranial haemorrhage - haematomas - cord bleeding ```
129
Ix for haemophillia
Bleeding scores Coagulation factor assays Genetic testing
130
Mx of haemophillia
Replacement of clotting factors by IV infusion - prophylactically - in response to bleeding - can result in antibody formation against clotting factor - becomes ineffective Desmopressin - stimulates release of von Willebrand factor Antifibrinolytics
131
Risk factors for VTE
``` Immobility Recent surgery Long haul flights Pregnancy Hormone therapy with oestrogen (combined oral contraceptive pill and hormone replacement therapy) Malignancy Polycythaemia Systemic lupus erythematosus Thrombophilia ```
132
DVT presentation
``` Unilateral Calf or leg swelling Dilated superficial veins Tenderness to the calf (particularly over the site of the deep veins) Oedema Colour changes to the leg ```
133
Ix for VTE
``` Wells score D-dimer - also raised - pneumonia - malignancy - heart failure - surgery - pregnancy USS doppler of leg CTPA or VQ for PE ```
134
Mx of VTE
Initial management with LMWH - started immediately before confirming diagnosis Long term anticoagulation - warfarin, NOAC (apixaban, dabigatran or rivaroxaban) or LMWH Continue anticoagulation for - 3 months in obvious reversible cause - beyond 3 months is cause unclear, recurrent VTE or irreversible underlying cause - 6 months is active cancer
135
Ix for unprovoked DVT
``` Screen for cancer - history + examination - CXR - bloods - FBC, calcium + LFTs - urine dipstick - CT abdo and pelvis in patients over 40 - Mammogram in women over 40 Antiphospholipid antibodies to check for antiphospholipid syndrome If FHx test for hereditary thrombophilias ```
136
Define Budd-Chiari syndrome
Blood clot develops in hepatic vein blocking outflow of blood -> acute hepatitis
137
Features of Budd-Chiari syndrome
Abdo pain Hepatomegaly Ascites
138
Presentation of ALL
Pancytopenia | Bone pain
139
Mx of ALL
Multi-drug chemotherapy
140
Complications of CLL
Infiltration of BM Reduction in repertoire of antibodies Altered T cell immunity Autoimmune phenomena - due to immune dysregulation - AIHA - ITP Transformation to high grade lymphoma - Richters transformation
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Mx of CLL
Not all need treatment - undergo watch and wait period Treatable but not curable Treated when clinically indicated • Hb<10 • Plt<100 • Significant lymphadenopathy, massive splenomegaly • Doubling time <6 months (time for lymphocyte count to double, only significant once the lymphocyte count is more than 30) • Infections • Autoimmune phenomena Treatment includes chemotherapy, targeted therapies such as monoclonal antibodies and tyrosine kinase inhibitors
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Secondary causes of high platelets
``` Bleeding Iron deficiency Inflammation Infection Post-surgery Splenectomy ```
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Secondary causes of high RBC
``` Chronic hypoxia Smoking Obstructive sleep apnoea Altitude Renal/hepatic tumours Congenital heart disease ```
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Role of lymphatic system
Defence against infection Return of tissue fluid to blood Absorption of fats and vitamins from GI tract
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Define aplastic anaemia
Failure of all lines resulting in pancytopenia - anaemia - thrombocytopenia - neutropenia
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Clinical features of aplastic anaemia
Anaemia Bleeding Infection
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Ix for aplastic anaemia
``` FBC Reticulocyte count B12 and folate levels Infection screen LFTs and U+Es Thyroid function Bone marrow aspirate for diagnosis ```
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Mx of aplastic anaemia
Supportive - reduce infection risk - treat infection aggressively - platelet and red cell transfusions as appropriate - ATG and ALG immunotherapy
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Causes of splenomegaly
``` Massive - myeloproliferative disorders - CML, AML and myelofibrosis - tropical infections - malaria - HIV Moderate - myeloproliferative disorders - lymphoproliferative disorders - CLL, lymphoma - amyloidosis Mild - myelo and lymphoproliferative disorders - portal hypertension - infections - IE, EBV, viral hepatitis - haemolytic anaemia - autoimmune - SLE ```

Yr4 - Cancer Care (12 decks)

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